髓磷酯髓鞘蛋白1抗体
规格:1mg/1ml
英文名: Myelin PLP
别名: Lipophilin; HLD1; Lipophilin; Major myelin proteolipid; Major myelin proteolipid protein; MMPL; Myelin proteolipid protein; MYPR_HUMAN; PLP 1; PLP; PLP/DM20; PLP1; PLP1 protein; PMD; Proteolipid prote
分子量: 30kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human Myelin p
交叉反应:Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Monkey,
细胞定位:细胞膜
髓磷酯髓鞘蛋白1抗体产品介绍:background: PLP is a major constituent of myelin. The two isoforms of the myelin proteolipid protein, PLP and DM20, are very hydrophobic integral membrane proteins that account for about half of the protein content of ***** CNS myelin. A mutation in the gene which encodes PLP is linked to Pelizaeus-Merzbacher disease (PMD), a chronic infantile type of diffuse cerebral sclerosis. The gene which encodes PLP maps to human chromosome Xq22. The glycoprotein zero (also designated P-zero or myelin peripheral protein) is the primary structural protein of peripheral myelin, and accounts for more than 50% of the protein present in the peripheral nerve sheath. Zero is an integral membrane glycoprotein. Expression of zero is restricted to Schwann cells. The gene which encodes zero maps to human chromosome 髓磷酯髓鞘蛋白1抗体1q22. PMP22 (peripheral myelin protein 22) is a growth-regulated membrane protein which is expressed by Schwann cells and is localized primarily in compact peripheral nervous system myelin. The gene which encodes PMP22 maps to human chromosome 17p11.2. Function: This is the major myelin protein from the central nervous system. It plays an important role in the formation or maintenance of the multilamellar structure of myelin. Subunit: Belongs to the myelin proteolipid protein family. Subcellular Location: Membrane; Multi-pass membrane protein. DISEASE: Defects in PLP1 are the cause of leukodystrophy hypomyelinating type 1 (HLD1) [MIM:312080]; also known as Pelizaeus-Merzbacher disease. HLD1 is an X-linked recessive dysmyelinating disorder of the central nervous system in which myelin is not formed properly. It is characterized clinically by nystagmus, spastic quadriplegia, ataxia, and developmental delay. Defects in PLP1 are the cause of spastic paraplegia X-linked type 2 (SPG2) [MIM:312920]. SPG2 is characterized by spastic gait and hyperreflexia. In some patients, complicating features include nystagmus, dysarthria, sensory disturbance, mental髓磷酯髓鞘蛋白1抗体 retardation, optic atrophy. Similarity: Belongs to the myelin proteolipid protein family. Gene ID: 5354 Database links: Entrez Gene: 281410 Cow Entrez Gene: 481002 Dog Entrez Gene: 5354 Human Entrez Gene: 18823 Mouse Entrez Gene: 24943 Rat Omim: 300401 Human SwissProt: P04116 Cow SwissProt: P23294 Dog SwissProt: P60201 Human SwissProt: P60202 Mouse SwissProt: P60203 Rat Unigene: 49691 Cow Unigene: 1787 Human Unigene: 1268 Mouse Unigene: 4550 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
髓磷酯髓鞘蛋白1抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:神经生物学 细胞粘附分子 细胞类型标志物 细胞膜蛋白
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid